A Late Onset of Adrenocortical Cancer Assosiated with Beckwith-Wiedemann Syndrome

Cover Page


Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder involving a predisposition to tumor development. The common features of Beckwith-Wiedemann syndrome include omphalocele, macroglos- sia and macrosomia. The increased risk for neoplasia is concentrated in the first eight years of life. However, this case presents a late onset of adrenocortical cancer assosiated with Beckwith-Wiedemann syndrome.

About the authors

N S Kuznetsov

Endocrinology Research Centre

N V Latkina

Endocrinology Research Centre

N Yu Kalinchenko

Endocrinology Research Centre

L S Selivanova

Endocrinology Research Centre

A O Raykhman

Email: toda_mi_alma@inbox.ru
I.M. Sechenov First Moscow State Medical University


  1. Wiedemann HR. Familial malformation complex with umbilical hernia and macroglossia - a “new syndrome”? J Genet Hum. 1964;13:223-232.
  2. Weksberg R, Shuman C, Bruce Beckwith JB. Beckwith- Wiedemann syndrome. Eur J Hum Genet. 2010;18(1): 8-14.
  3. Мельниченко Г.А., Петеркова В.А., Тюльпаков А.Н., Максимова Н.В. Эпонимические синдромы в эндокринологии. М., 2013.
  4. Cooper WN, Luharia A, Evans GA et al. Molecular subtypes and phenotypic expression of Beckwith-Wiedemann syndrome. Eur J Hum Genet. 2005;13:1025.
  5. Reish O, Lerer I, Amiel A et al. Wiedemann-Beckwith syndrome: further prenatal characterization of the condition. Am J Med Genet. 2002;107(3):209-213.
  6. Halliday J, Oke K, Breheny S et al. Beckwith-Wiedemann syndrome and IVF: a case-control study. Am J Hum Genet. 2004;75(3):526-528.
  7. Hertel NT, Carlsen N, Kerndrup G et al. Late relapse of adrenocortical carcinoma in Beckwith-Wiedemann syndrome. Clinical, endocrinological and genetic aspects. Acta Paediatr. 2003;92(4):439-443.
  8. Sbragia-Neto L, Melo-Filho AA, Guerra-Junior G et al. Beckwith-Wiedemann syndrome and virilizing cortical adrenal. tumor in a child. J Pediatr Surg. 2000;35(8):1269-1271.
  9. DeBaun MR. Screening Protocol for Beckwith-Wiedemann Syndrome. Beckwith-Wiedemann Children’s foundation.



Abstract - 1764

PDF (Russian) - 1242



Copyright (c) 2014 ., ., ., ., .

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies